Shapiro J M, Smith H, Schaffner F
Gut. 1979 Feb;20(2):137-40. doi: 10.1136/gut.20.2.137.
We followed up 55 patients with proven primary biliary cirrhosis for several years or until death. A graph of the level of serum bilirubin versus time that was constructed for each patient shows an initial period of variable length in which the serum bilirubin level remained constant. This was followed by a period of rapid rise in serum bilirubin which culminated in the patient's death. Whenever two successive serum bilirubin values taken six months apart exceeded 34 mumol/l (2.0 mg/dl) the patient had entered a late phase of disease and lived an average of 49 months. Ninety-five per cent confidence limits on survival time were 32-74 months. If two successive six month bilirubin values exceeded 102 mumol/l (6.0 mg/dl), calculated survival time was 25 months, and if two successive six month bilirubin values exceeded 170 mumol/l (10.0 mg/dl), survival time was 17 months. Fifteen of the 41 living patients had two consecutive serum bilirubin levels greater than 34 mumol/l (2.0 mg/dl). However, the slope of the rising bilirubin in the living patients is only 35 mumol/l/yr (1.5 mg/dl/yr) compared with 42 mumol/l/yr (2.5 mg/dl/yr) in the dead patients. This means that patients with this disease not may be living considerably longer.
我们对55例经证实的原发性胆汁性肝硬化患者进行了数年随访,直至其死亡。为每位患者绘制的血清胆红素水平随时间变化的图表显示,在一段长短不一的初始期内,血清胆红素水平保持稳定。随后是血清胆红素迅速上升的时期,最终导致患者死亡。每当相隔6个月的连续两次血清胆红素值超过34μmol/L(2.0mg/dl)时,患者就进入了疾病晚期,平均存活49个月。生存时间的95%置信区间为32 - 74个月。如果相隔6个月的连续两次胆红素值超过102μmol/L(6.0mg/dl),计算得出的生存时间为25个月;如果相隔6个月的连续两次胆红素值超过170μmol/L(10.0mg/dl),生存时间为17个月。41例存活患者中有15例连续两次血清胆红素水平大于34μmol/L(2.0mg/dl)。然而,存活患者胆红素上升的斜率仅为35μmol/L/年(1.5mg/dl/年),而死亡患者为42μmol/L/年(2.5mg/dl/年)。这意味着患有这种疾病的患者可能不会存活更长时间。